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Autoimmune blistering skin diseases (AIBD) encompass a group of diseases characterised by cutaneous and/or mucocutaneous fragility. Multiple risk factors contributing to osteoporosis exist in AIBD patients, including use of long‐term systemic corticosteroid therapy (SCT), decreased mobility and the presence of chronic inflammation. Despite this, there is no consensus on the prophylaxis of osteoporosis in AIBD, especially in the absence of SCT. To systemically review the current literature on the association between osteoporosis/osteopenia and AIBD, a comprehensive literature search was performed on six online databases with search terms related to bone mineral density (BMD) and AIBD. A total of 314 articles were screened by their abstract and/or full text. A total of 20 peer‐reviewed full‐text articles addressing BMD in patients with AIBD were identified. Eight articles examined the association between osteoporosis and pemphigus. Only two articles examined the association between osteoporosis and pemphigoid patients. Three articles examined the effectiveness of osteoporosis prophylaxis in AIBD patients. Seven papers examined the levels of vitamin D in AIBD patients. Few case‐control studies examine osteoporosis in pemphigus in the context of SCT, with consistent findings. However, there is scarce literature examining the risk of osteoporosis in pemphigoid, or in AIBD without SCT. Prophylaxis and screening of osteoporosis in AIBD is suboptimal and more attention in this area is required to avoid future complications related to osteopenia and osteoporosis. [ABSTRACT FROM AUTHOR]

Background: The BIOCHIP is a novel multiplex indirect immunofluorescence technique used in the serological diagnosis of bullous pemphigoid and pemphigus. The BIOCHIP method combines the screening of autoantibodies and target antigen‐specific substrates in a single miniature incubation field. Objective: To evaluate the diagnostic accuracy of the new immunofluorescence BIOCHIP multiplex tool in pemphigus and bullous pemphigoid. Methods: For the validation of the BIOCHIP, sera from patients with BP (n = 38), PF (n = 8) and pemphigus vulgaris (PV) (n = 23) were used. In addition, sera from disease control patients (n = 63) and healthy volunteers (n = 39) were used. The multiplex BIOCHIP and direct immunofluorescence (DIF) were performed for all BP, PF and PV patients. Additional indirect immunofluorescence (IIF) was performed on patients with BP, and ELISA was performed on patients with pemphigus. Results: The BIOCHIP mosaic showed a sensitivity of 86.8% and specificity of 85% for BP180 or BP230 being positive in BP. It demonstrated a sensitivity of 75% and specificity of 97.7% for Dsg1 in PF. The BIOCHIP was found to have a sensitivity of 60.9% and specificity of 73.6% for Dsg3 in PV. Conclusion: The BIOCHIP mosaic‐based immunofluorescence test is potentially a simple, time and effort saving test that can aid in the diagnosis and screening of BP, PV and PF. However, there is potential for interpretation bias and a learning curve that needs to be taken into consideration. [ABSTRACT FROM AUTHOR]

Background: The BIOCHIP (Dermatology Mosaic 7; EUROIMMUN, Lubeck, Germany) is a novel multiplex indirect immunofluorescence (IIF) technique used in the serological diagnosis of bullous pemphigoid (BP) and pemphigus. Objective: To validate the accuracy and inter‐rater reliability (IRR) of the BIOCHIP in the diagnosis of BP, pemphigus foliaceus (PF) and pemphigus vulgaris (PV). Methods: Sera from patients with BP (n = 38), PF (n = 8), PV (n = 23), control patients (n = 64) and healthy control volunteers (n = 39) were tested. Sera were collected and analysed during the course of the disease at 1–5 different time points. The BIOCHIP was performed for all patients, digital images were captured of each incubated field, and the images were shared with 10 dermatologists experienced in reading IF from around the world to report. There were 312 BIOCHIP slides consisting of 1872 photos in total. All patients were de‐identified. Fleiss Kappa was used to estimate the IRR. Results: Fleiss Kappa was computed for each category (Oesophagus, Oesophagus immunofluorescence pattern, Salt‐Split Skin (SSS), SSS immunofluorescence location, BP180, BP230, Dsg 1 and Ds3). The inter‐rater agreement between the 10 raters varied between fair and moderate for all categories. Those that demonstrated fair concordance included monkey oesophagus (k = 0.257, P < 0.0001), oesophagus pattern (k = 0.357, P < 0.0001), Dsg1 (k = 0.390, P < 0.0001) and BP230 (k = 0.281, P < 0.0001). Moderate agreement was demonstrated for SSS (k = 0.416, P < 0.0001), SSS immunofluorescence location (k = 0.505, P < 0.0001), Dsg3 (k = 0.437, P < 0.0001) and BP180 (k = 0.559, P < 0.0001). Conclusion: The BIOCHIP mosaic‐based immunofluorescence test is a simple, time and effort saving test that can aid in the diagnosis and screening of BP, PV and PF. However, the level of agreement was relatively low. The authors found the most common causes to be variable levels of training, indicating the presence of a learning curve in the interpretation of the results and ambiguous staining patterns leading to incongruent results. [ABSTRACT FROM AUTHOR]

The SARS-CoV-2 pandemic has worsened since the publication of our initial recommendations for the management of autoimmune bullous diseases (AIBDs) during the COVID-19 outbreak in April 2020.1 Based on the rapidly emerging increase in knowledge, this consensus of an expanded panel of international AIBD experts proposes updated recommendations to promote the optimal care of AIBD patients during the pandemic. Temporary changes in some immunosuppressive medications are still recommended in patients who have active COVID-19, as detailed in our previously suggested guidelines.1 B What should patients with AIBDs do to protect themselves from SARS-CoV-2 infection? b Patients with AIBDs treated with immunosuppressive therapies are generally prone to develop opportunistic infections,2,3 which raised concerns that they could be more susceptible to SARS-CoV-2 infection and/or have worse COVID-19 outcomes. [Extracted from the article]

Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub‐epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. The classification and management is based on clinical, histological and direct and indirect immunofluorescence findings. There are, however, overlapping clinical and histological features between the conditions and clinical heterogeneity within each disease. [ABSTRACT FROM AUTHOR]

Background Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score ( ABSIS) and Pemphigus Disease Area Index ( PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate, significant and extensive types of pemphigus. Objectives To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores. Methods In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment ( PGA) and Dermatology Life Quality Index ( DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups. Results Ninety-six patients with pemphigus vulgaris ( n = 77) or pemphigus foliaceus ( n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS ( P < 0·001), PDAI ( P < 0·001), PGA ( P < 0·001) and DLQI ( P = 0·03) scores. Conclusions This study suggests cut-off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus. [ABSTRACT FROM AUTHOR]

Autoimmune blistering diseases ( AIBD) are a group of rare but potentially fatal diseases characterized by the production of autoantibodies directed against the structural proteins of the skin. Much has been published on the clinical manifestation and interventional options for AIBD, especially on the more common subtypes such as bullous pemphigoid, pemphigus vulgaris ( PV) and pemphigus foliaceus ( PF). However, the aetiology of AIBD remains unknown. We aim to provide an overview of published case-control studies focussing on the non-genetic aetiological factors of bullous pemphigoid, PV and/or PF. The relevant studies were appraised for their validity and results. Our results showed that a large proportion of the studies had inconclusive results due to compromised study methodologies. Moreover, there were no identified case-control studies that investigated the possible associations between bullous pemphigoid and patient environment, or the potential links between pemphigus and drugs. Hence, a case-control study with a higher quality design addressing these shortcomings would contribute greatly to our knowledge of AIBD. [ABSTRACT FROM AUTHOR]

The article focuses on a study related to relationship of bullous pemphigoid (BP) with different neurological diseases such as multiple sclerosis. Topics discussed include cross-reactivity towards epithelial and neuronal isoforms leads to autoantibodies development in BP through adhesion complex BPAG-1; analysis of risks related to prevalence of MS among BP patients; and consideration of Dimethyl fumarate usage for treatment of BP with approval of the European Medicines Agency.

The article presents an editorial on a research paper "Hospitalization, Inpatient Burden and Comorbidities Associated With Bullous Pemphigoid (BP) in the U.S.A.," by researcher Z. Ren and colleagues. Topics discussed include an increased prevalence of BP in elderly people, factors playing important role in hospitalizations in BP and need for safer and more specific therapies to manage patients with BP.